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Colegio de Medicos y Cirujanos República de Costa Rica

2015, Número 617

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Rev Med Cos Cen 2015; 72 (617)


Sarcoma de Ewing

Villalta FJC

Idioma: Español
Referencias bibliográficas: 33
Paginas: 695-704
Archivo PDF: 413.25 Kb.


RESUMEN

El Sarcoma de Ewing pertenece a un grupo de enfermedades neoplásicas conocidas como la familia de tumores de Ewing,los cuales incluyen además su variante extraósea, el tumor neuroectodérmico, el tumor de Askin y los tumores atípicos de Ewing. Su origen ha sido debatido por largo tiempo e incluso se le ha señalado un posible origen endotelial y vascular como el que le confirió James Ewing en 1921. La verdad de lo anterior es que existe prueba científica suficiente desde el punto de vista inmunohistoquímico, citogenético y de estudios moleculares genéticos que apoyan el origen mesenquimático de una célula progenitora común para todos los tumores de la familia de los sarcomas de Ewing. El Sarcoma de Ewing se caracteriza genéticamente por presentar en la gran mayoría de los casos una anomalía citogenética, la cual se trata de un trastorno cromosómico asociado a una translocación del cromosoma 22 t(11:22) (q24:q12). La familia de tumores de Ewing se origina por lo general en los huesos largos de las extremidades, principalmente en el fémur, pero también en la tibia, fíbula, húmero y en los huesos de la pelvis. Usualmente son menos afectados los huesos de la columna vertebral, manos y pies. Se reporta fundamentalmente en hombres con una relación hombre/mujer de 1:6. Tiene una prevalencia de 1-5 casos por cada 1.000.000 de habitantes dándose el 70% en menores de 20 años (entre 11-12 años en niñas, entre 15-16 años en niños.


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